Endothelial activation and increased heparan sulfate expression in cystic fibrosis.

Rationale: Pulmonary disease in cystic fibrosis (CF) is characterized by an exaggerated interleukin (IL)-8-driven, neutrophilic, inflammatory response to infection. Binding of IL-8 to heparan sulfate (HS)-containing proteoglycans (HSPG) facilitates binding of the chemokine to its specific receptor, stabilizes and prolongs IL-8 activity, and protects it from proteolysis. We hypothesized that increased expression of HSPG contributes to the sustained inflammatory response in CF bronchial tissue.